Spinal Cord (2005) 43, 249–251

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Case Report

Foramen magnum decompression for the treatment of Arnold Chiari

malformation type I with associated syringomyelia in an elderly patient
I Takigami1, K Miyamoto*,1, H Kodama1, H Hosoe1, S Tanimoto2 and K Shimizu1
1
Department of Orthopaedic Surgery, Gifu University School of Medicine, Gifu City, 501-1194, Gifu, Japan;
2
Bicchu Orthopaedic Hospital, Nariwa Cho, Kawakami-Gun, Okayama, Japan

Study design: Report of a rare case of an elderly patient with late onset of Arnold Chiari
malformation type I with associated syringomyelia that was successfully treated with foramen
magnum decompression.
Objective: To report this rare case along with a literature review.
Setting: Gifu, Japan.
Methods: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred
to the clinic. After physical and radiographical examinations, she was diagnosed with Arnold
Chiari malformation type I with associated syringomyelia. A foramen magnum decompression
by the removal of the outer layer of the dura mater was performed.
Results: At 2 years postoperatively, MRI revealed a decrease in the size of the syringomyelia.
Her symptoms had also remarkably improved.
Conclusions: A rare case of symptomatic Arnold Chiari malformation type I with associated
syringomyelia in an elderly woman was successfully treated with foramen magnum
decompression by the removal of the outer layer of the dura mater.
Spinal Cord (2005) 43, 249–251. doi:10.1038/sj.sc.3101675; Published online 2 November 2004

Keywords: Arnold Chiari malformation type I; syringomyelia; foramen magnum decompres-

sion; elderly; surgical treatment

Introduction
In Arnold Chiari malformation type I, abnormal
cerebrospinal fluid flow occurs and frequently results
in syringomyelia.1,2 Various surgical methods for treat-
ing neurologically symptomatic cases of syringomyelia
associated with Arnold Chiari malformation type I
have been reported.1,3–7 Posterior fossa craniectomy
(foramen magnum decompression), with1 or without
plugging of the obex,3,8,9 has been one of the most
commonly performed surgical procedures in the belief
that it would ameliorate the abnormal cerebrospinal
fluid flow. However, this surgical procedure has been
primarily indicated for treating relatively young and
middle-aged patients.1,3,4,6,10 Few reports are available
on the late onset of symptoms associated with Arnold
Chiari malformation type I in elderly patients and
possible surgical treatments.8,11 This report is of a very
rare case of an elderly woman with late-onset Arnold
Chiari malformation type I and syringomyelia that
*Correspondence: K Miyamoto, Department of Orthopaedic Surgery,
Gifu University School of Medicine, Gifu City, 501-1194, Gifu, Japan
was successfully treated with foramen magnum decom-
pression.
Case report
In May 2000, a 69-year-old woman was referred to the
clinic. She had experienced a history of dull pain in her
right arm for 4 years. Additionally, dorsalgia and
bilateral leg pain radiating from her gluteal region
appeared 1 year prior to her evaluation. Owing to the
dorsalgia and leg pain, her gait became gradually
disturbed. On her first visit to the clinic, the neurological
examination revealed sensory disturbances in her right
arm, trunk and thigh. Deep tendon reflexes were
depressed in the right arm and exaggerated in both legs.
Plain X-ray of the cervical spine showed moderate
degenerative changes. MRI showed cerebellar tonsillar
herniation into the foramen magnum and syringomyelia
from the medulla oblongata to the T11 level (Figure 1).
However, compression of the spinal cord due to
spondylotic change or disc herniation at the cervical
spine level was not observed. Therefore, she was
 Successful foramen magnum decompression for an elderly patient
I Takigami et al
250
Figure 1 Preoperative T1-weighted MRI scan of the occipi-
tocervical junction and the cervical spine. Sagittal section just
at the midline of the spinal cord demonstrates syringomyelia
from medulla oblongata to upper thoracic levels
diagnosed with symptomatic Arnold Chiari malforma-
tion type I with associated syringomyelia.
Surgical procedure
Foramen magnum decompression by the removal of
the outer layer of the dura mater was performed, as
reported by Isu et al6 in 1993. First, a suboccipital
craniectomy and removal of the arch of C1 was
performed. It is worth noting that in a suboccipital
craniectomy, the occipital bone is removed far laterally.6
A cruciate incision was made in the outer layer of the
dura mater. The outer layer was dissected from the inner
layer and removed. Immediately after this removal, the
inner layer of the dura mater began bulging and the
cerebellar tonsil was pulsating. Therefore, the effective-
ness of decompression at the foramen magnum was
immediately ascertained.
At 1 month postoperatively, the sensory disturbances
in her right arm and both legs improved and her
difficulty in walking disappeared. The dorsalgia and
bilateral leg pain radiating from her gluteal region were
remarkably reduced. No perioperative complications
occurred. In the postoperative MRI, both the upper
deviation of the cerebellar tonsil and a reduction in size
of the syrinx were recognized (Figure 2). After a 2-year
follow-up, her postoperative course has been excellent.
Spinal Cord
Figure 2 Postoperative T1-weighted MRI scan of the
occipitocervical junction and cervical spine. Sufficient decom-
pression at the foramen magnum, upper deviation of cerebeller
tonsil and remarkable reduction in size of the syringomyelia
are shown
Discussion
According to the literature, the clinical condition of this
patient is doubly rare and worth reporting. First,
because the Arnold Chiari malformation type I with
associated syringomyelia initially manifested its symp-
toms at an advanced age, and second, because a
satisfactory surgical outcome following foramen
magnum decompression was achieved in an elderly
patient.
There are several reports on symptomatic Arnold
Chiari malformation type I associated with syringomye-
lia.1,3,8–10,12,13 Among these, a few authors presented
cases with late onset of symptoms due to Arnold Chiari
malformation type I-associated syringomyelia.8,12 Mor-
iwaka et al14 reported that the average age of onset of
the first symptoms due to syringomyelia was 28 -years
old and that syringomyelia due to an Arnold Chiari
malformation showed a tendency to have a younger age
of onset.
In the case reported here, both MRI and CT-
myelogram revealed that there was no compression of
the spinal cord due to degenerative changes in the
cervical spine. Therefore, foramen magnum decompres-
sion was chosen to treat the patient, with the expectation

that this procedure would improve the cerebrospinal
fluid flow and alleviate her symptoms.
There is apparently only one other report, by Geroldi
et al,11 of the successful surgical treatment of an elderly
patient using foramen magnum decompression for
Arnold Chiari malformation type I with associated
syringomyelia. That case differs from the present one
in that a cervico-spinal decompression was performed,
although the surgical procedure was not precisely
described. In the current case, because there was no
observable compression of the cervical spinal cord,
foramen magnum decompression by the removal of the
outer layer of the dura mater6 was performed. Among
the various methods for foramen magnum decompres-
sion, this method has several advantages. First, it is less
invasive in comparison with other previously reported
procedures. Second, the reported incident rate of read-
hesion is low, and third, the decompression effect is
maintained for a long period because the inner layer of
the dura mater is preserved.15 In addition, because this
foramen magnum decompression procedure is extra-
dural, there are no demonstrated risks of such post-
operative complications6 as cerebrospinal fluid collec-
tion in the operative wound,4 pseudomeningocele10 or
meningitis.9,16 Therefore, the authors are convinced that
this method is advantageous when performing a fora-
men decompression in an elderly patient.
In a report by Isu et al,6 the oldest patient who
underwent this procedure was 54 years old. This patient
presented with symptoms induced by a cervical lesion at
65 years of age. In elderly patients, cervical myelopathy
is encountered frequently in clinical practice and is
difficult to differentiate from other conditions by a
physical examination and plain X-ray alone. The
symptoms of Arnold Chiari malformation type I with
associated syringomyelia vary and this clinical condition
has no pathognomonic clinical sign.8,17 In the current
patient, the MRI scan from the occipitocervical junction
down to the cervical spine was very useful in making a
correct diagnosis. Moreover, the MRI scan revealed the
postoperative reduction of the syringomyelia, which
corresponded well with the alleviation of her symptoms.
Awareness of symptomatic Arnold Chiari malformation
type I-associated syringomyelia in elderly patients would
help clinicians make correct diagnoses.
Conclusion
A rare case of Arnold Chiari malformation type I
associated with syringomyelia was reported in an elderly
woman, who presented with sensory disturbances and
pain in her right arm, trunk and both legs. She was
successfully treated by foramen magnum decompression
by the removal of the outer layer of the dura mater.
Successful foramen magnum decompression for an elderly patient
I Takigami et al
251
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